0000000000362331
AUTHOR
S. Berger
The clinical relevance of non-classified dysganglionoses and implications for a new grading system
In addition to the classified types of dysganglionosis, certain non-classified dysganglionoses (NCD) (types 1-4) were introduced by Meier-Ruge in 1992. Clinical data on these conditions are limited. Among 134 children with intestinal dysganglionoses (ID) treated from 1979 to 1999, 12 were identified to have a NCD. Their clinical course is presented. The existence of mild ID (type 1) is difficult to demonstrate. Current definitions and data on clinical relevance are not convincing. An indication for surgical treatment is not present. Isolated hypogenesis of the submucous plexus (SMP) (type 2, n = 8) is clinically a more severe kind of intestinal neuronal dysplasia type B and often requires e…
Complex long-segment intestinal dysganglionosis.
A case is reported with aganglionosis of the rectum, sigma, and descending colon; dysganglionosis with heterotopic ganglionic cells in the muscularis propria of the hypoganglionic transverse colon; and extreme hypoganglionosis (without detection of ganglionic cells) of the ascending colon and distal ileum. The ileum showed a transition zone with hypoganglionosis and intestinal neuronal dysplasia (IND) type B. As to the etiology of such complex intestinal innervation defects, pre- and perinatal perfusion deficits must be considered because their localization seems to be linked to the vascular anatomy of the colon. Early diagnosis may be difficult, causing a delay in operative treatment and m…
Congenital malformations and perinatal morbidity associated with intestinal neuronal dysplasia.
A close relation between different forms of dysganglionosis such as intestinal neuronal dysplasia (IND) type B and aganglionosis has been established. No systematic analysis of other malformations and diseases accompanying IND has been made as yet. Congenital malformations and perinatal morbidity were analyzed in 109 patients with IND seen at the Department of Pediatric Surgery in Mainz from 1977 to 1996. IND was associated with Hirschsprung's disease in 47 cases; 22 children with IND had other abdominal malformations, including anal atresia, rectal stenosis, sigmoidal stenosis, ileal atresia, pyloric stenosis, and esophageal atresia. A cystic bowel duplication, a choledochal cyst, and a pe…
Relationship of Cerebral Blood Flow Disturbances with Brain Oedema Formation
Brain oedema is an important factor which compromises maintenance of the cerebral blood flow. Conversely, primary blood flow disturbances are leading to brain oedema. The mechanisms underlying blood flow impairment by brain oedema are associated with an increased regional tissue pressure in proportion to the degree of water accumulation in the parenchyma. The release of vasoactive mediator compounds might be considered in addition. Primary disturbances of the cerebral blood flow, such as focal or global cerebral ischaemia are leading to an increased cerebral water content. A decrease of the cerebral blood flow to ca. 40% of normal or below has been found to result in the development of brai…
Treatment of congenital abdominal wall defects —a 25-year review of 132 patients
The outcome data of 132 patients treated at the Department of Pediatric Surgery in Mainz during the last 25 years were reviewed. Prenatal diagnosis of abdominal wall defects (AWDs) and associated malformations led to increasing selection of the patient population. The aim of primary closure of the abdominal wall can be achieved more frequently in gastroschisis (GS) than omphalocele (OC), while the postoperative course is more complicated and of longer duration in GS. Delayed or secondary closure extended the hospitalization period but had no negative effects on the outcome. Reoperations or planned secondary operations were performed in 23 patients with GS and 14 with OC. Early mortality was…
Postoperative intussusception in childhood
Over a period of 10 years, five children developed postoperative intussusception after intra-abdominal procedures at the Department of Pediatric Surgery of the Johannes Gutenberg University Mainz. Two appendectomies, one ileal resection for a Meckel's diverticulum, one operative procedure for Hirschsprung's disease plus intestinal neuronal dysplasia type B, and one hiatoplasty with jejunostomy preceded the intussusception. Three of the five children were older than 2 years. The clinical symptoms consisted primarily of abdominal distension, diffuse abdominal pain, bilious vomiting, and rectal bleeding in one case. Preoperative diagnosis was achieved in four cases by abdominal ultrasound. Pla…
Injuries of the lateral collateral ligaments of the ankle: assessment with MR imaging.
The aim of this study was to evaluate the ability of MRI to display injuries of the lateral collateral ligamentous complex in patients with an acute ankle distorsion trauma. The MR examinations of 36 patients with ankle pain after ankle distorsion were evaluated retrospectively without knowledge of clinical history, outcome and/or operative findings. The examinations were performed on a 1. 5-T whole-body imager using a flexible surface coil. The signs for ligamentous abnormality were as follows: complete or partial discontinuity, increased signal within, and irregularity and waviness of the ligament. The results were compared with operative findings in 18 patients with subsequent surgical r…
Die Rolle des Kinderchirurgen bei der klinischen Versorgung des Kindes mit Schädel-Hirn-Trauma
Die Versorgung einer schweren Schadel-Hirn-Verletzung im Kindesalter ist eine multidisziplinare Aufgabe. Die besonderen Aufgaben des Kinderchirurgen in der Behandlung des schweren Schadel-Hirn-Traumas (SHT) liegen in der Diagnostik und Therapie der haufig vorhandenen extrakraniellen Verletzungen, was fur die Prognose des Patienten von entscheidender Bedeutung sein kann. Ziel ist hier insbesondere die Vermeidung bzw. Behandlung einer systemischen Hypotension und Hypoxie, um damit der Entwicklung sekundarer Hirnschaden entgegenzuwirken. Die Behandlung, bzw. Diagnostik und Uberwachung der leichteren Schadel-Hirn-Verletzungen ist ein weiterer Schwerpunkt des Kinderchirurgen. Weiterhin besteht e…
Patient participation in the medical decision-making process in haemato-oncology - a qualitative study
Cancer patients are showing increased interest in shared decision-making. Patients with haematological illnesses, however, express considerably less desire for shared decision-making as compared with other oncological patient groups. The goal of the current project was to identify the reasons for the lower desire for shared decision-making among patients with haematological illness. We conducted qualitative, semi-structured interviews with 11 haematological patients (39-70 years old) after the beginning of therapy concerning the course and evaluation of medical shared decision-making. The patients were often overwhelmed by the complexity of the illness and the therapy and did not want to as…
Hundebissverletzungen des Gesichtes bei Kindern
Hundebissverletzungen betreffen im Kindesalter besonders haufig den Gesichts- und Nackenbereich. Diese stark infektionsgefahrdeten Wunden verlangen wegen ihrer in funktioneller und kosmetischer Hinsicht wichtigen Lokalisation eine spezielle kinderchirurgische Behandlung. Das beste kosmetische Ergebnis wird durch eine primare chirurgische Wundversorgung erreicht, diese wird in Abhangigkeit vom Verletzungsausmas in Allgemeinnarkose durchgefuhrt. Trotz der niedrigen Inzidenz von Rabiesinfektionen und Tetanusinfektionen ist die genaue Abklarung des Impfstatus von Kind und Hund von zentraler Bedeutung, da die manifesten Infektionen einen letalen oder zumindest lebensgefahrlichen Verlauf nehmen. …